ABSTRACT
A Síndrome de Vohwinkel ou ceratodermia hereditária mutilante é uma ceratodermia palmoplantar rara, que se manifesta na infância e se torna mais evidente nas fases de adolescência e idade adulta. Doença de herança preferencialmente autossômica dominante, acomete mais mulheres e caucasianos. A hiperceratose palmoplantar difusa, com aparência de favo de mel; as faixas constritivas digitais conhecidas como pseudoainhum, e as placas ceratósicas em forma de estrela-do-mar no dorso de mãos e pés, podendo acometer cotovelos e joelhos, são os achados clínicos característicos da Síndrome. O presente trabalho trata de um relato de caso de uma paciente com Síndrome de Vohwinkel e a terapêutica cirúrgica realizada nas faixas constritivas.
Vohwinkel syndrome, also known as hereditary mutilating keratoderma, is a rare palmoplantar keratoderma that manifests in childhood and becomes more evident in adolescence and adulthood. This preferential autosomal dominant disease affects more women and Caucasians. Its clinical features are diffuse palmoplantar keratoderma with the appearance of honeycomblike constricting rings in the fingers and toes known as pseudo-ainhum, and starfish-shaped keratotic plaques on the dorsal aspect of the hands and feet that can affect the elbows and knees. The present report describes a case report of a patient with Vohwinkel syndrome and surgical correction of the constrictive bands.
Subject(s)
Humans , Male , Adult , History, 21st Century , Retinoids , Callosities , Review Literature as Topic , Keratoderma, Palmoplantar , Transplants , Rare Diseases , Amputation, Surgical , Keratolytic Agents , Retinoids/therapeutic use , Retinoids/pharmacology , Callosities/surgery , Callosities/pathology , Keratoderma, Palmoplantar/surgery , Keratoderma, Palmoplantar/pathology , Transplants/surgery , Rare Diseases/history , Rare Diseases/pathology , Amputation, Surgical/adverse effects , Amputation, Surgical/methods , Keratolytic Agents/therapeutic use , Keratolytic Agents/pharmacologyABSTRACT
Background: Macrolides are prescribed in the treatment of pityriasis rosea despite conflicting results of the limited number of studies evaluating their role in its treatment. Aim: A randomized double-blind placebo-controlled trial was conducted to evaluate the effect of azithromycin on the clinical course of pityriasis rosea. Methods: Seventy patients of pityriasis rosea were given either azithromycin (n = 35) or placebo (n = 35) and were followed-up at 2, 4 and 6 weeks. Pruritus was assessed in both groups using the visual analogue scale (VAS) . Change in the pityriasis rosea severity score (PRSS) and in the VAS were recorded as outcome measures and were compared statistically. Results: The decrease in PRSS from baseline through 2, 4 and 6 weeks within both treatment (P < 0.001) and placebo (P < 0.001) arms was found to be statistically significant; however, this change was not significantly different in the two groups (P = 0.179). Similarly, the decrease in VAS was found to be statistically significant within both groups (P < 0.001); however, the change was comparable between the two groups (P < 0.937). Analysis by Fisher's exact test did not find a significant difference between the two groups for PRSS and VAS. Conclusion: Azithromycin is not effective in pityriasis rosea and the use of macrolides for this disease should not be encouraged in clinical practice.